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11.
Obesity-related focal and segmental glomerulosclerosis: normalization of proteinuria in an adolescent after bariatric surgery 总被引:1,自引:0,他引:1
Fowler SM Kon V Ma L Richards WO Fogo AB Hunley TE 《Pediatric nephrology (Berlin, Germany)》2009,24(4):851-855
Obesity-related glomerulopathy (ORG) is a secondary form of focal and segmental glomerulosclerosis (FSGS) occurring in severely
obese patients. A significant percentage of individuals with ORG will develop renal insufficiency or end stage renal disease.
We report here a 17-year-old girl with morbid obesity (body mass index 56.8 kg/m2) and ORG presenting with nephrotic range proteinuria, who failed to improve following treatment with diet, exercise and angiotensin-converting
enzyme inhibitor (ACEi)/angiotensin receptor blocker (ARB) therapy. Laparoscopic gastric bypass surgery was performed, and
within 2 weeks following the surgery, the patient had lost 5.7 kg body weight and showed a remarkable decrease in protein
excretion to one tenth of pre-surgery levels. More than 1 year after surgery, the patient’s urine protein and kidney function
have remained normal while off renin–angiotensin system inhibition therapy. This is the first report of successful use of
gastric bypass surgery for obesity-related glomerulopathy in an adolescent. We propose that gastric bypass surgery be considered
for patients with ORG. 相似文献
12.
Schwaderer P Knüppel T Konrad M Mehls O Schärer K Schaefer F Weber S 《Pediatric nephrology (Berlin, Germany)》2008,23(2):251-256
A small fraction of patients with initial steroid-sensitive nephrotic syndrome (SSNS) develops late steroid resistance, i.e.
a lack of remission after 4 weeks of relapse treatment despite previous response to steroids. The pathophysiological basis
of late resistance and the long-term prognosis remain obscure. Fourteen out of 360 patients with SSNS who were seen in our
department between 1954 and 2005 developed late resistance. Median age at onset of NS was 4 years and median duration of development
of late resistance 4.6 months. Histology showed minimal-change (MC) nephropathy in six patients and focal segmental glomerulosclerosis
(FSGS) in three patients on initial biopsy and four patients on repeat biopsies. Late resistance was treated with cyclophosphamide
in five patients, cyclosporine A in three, and both drugs in one. Eight of these nine patients went into remission. All 14
patients maintained a stable kidney function during their period of observation. NPHS2 mutation analysis in eight patients revealed no pathogenic mutations, suggesting that late resistance is not typically associated
with mutations in the NPHS2 gene. With respect to the clinical course, late resistance appears to resemble SSNS and is characterized by a favorable outcome. 相似文献
13.
Situs inversus totalis is a rare congenital anomaly that often occurs concomitantly with other disorders. A spectrum of renal abnormalities of patients with situs inversus has been reported. Developmental anomalies, including agenesis, dysplasia, hypoplasia, ectopia, polycystic kidney, and horseshoe kidney, have been reported. The association of situs inversus with nephrotic syndrome is very rare. We report the first known case of situs inversus totalis with nephrotic syndrome caused by primary focal segmental glomerulosclerosis, and the possible mechanism of this association. 相似文献
14.
Mary Carmelle Philogene Sheng Zhou Bonnie E. Lonze Serena Bagnasco Sami Alasfar Robert A. Montgomery Edward Kraus Annette M. Jackson Mary S. Leffell Andrea A. Zachary 《Human immunology》2018,79(4):195-202
Retrospective studies of angiotensin II type 1 receptor antibodies (AT1R-Ab) and anti-endothelial cell antibodies (AECA) have linked these antibodies to allograft injury. Because rising healthcare costs dictate judicious use of laboratory testing, we sought to define characteristics of kidney transplant recipients who may benefit from screening for non-HLA antibodies. Kidney recipients transplanted between 2011 and 2016 at Johns Hopkins, were evaluated for AT1R-Ab and AECA. Pre-transplant antibody levels were compared to clinical and biopsy indications of graft dysfunction. Biopsies were graded using the Banff' 2009–2013 criteria. AT1R-Ab and AECA were detected using ELISA and endothelial cell crossmatches, respectively. AT1R-Ab levels were higher in patients who were positive for AECAs. Re-transplanted patients (p?<?0.0001), males (p?=?0.008) and those with FSGS (p?=?0.04) and younger (p?=?0.04) at time of transplantation were more likely to be positive for AT1R-Ab prior to transplantation. Recipients who were positive for AT1R-Ab prior to transplantation had increases in serum creatinine within 3?months post-transplantation (p?<?0.0001) and developed abnormal biopsies earlier than did AT1R-Ab negative patients (126?days versus 368?days respectively; p?=?0.02). Defining a clinical protocol to identify and preemptively treat patients at risk for acute rejection with detectable non-HLA antibodies is an important objective for the transplant community. 相似文献
15.
Musab S. Hommos An S. De Vriese Mariam P. Alexander Sanjeev Sethi Lisa Vaughan Ladan Zand Kharmen Bharucha Nicola Lepori Andrew D. Rule Fernando C. Fervenza 《Mayo Clinic proceedings. Mayo Clinic》2017,92(12):1772-1781
Objectives
To describe the change in the incidence rates of primary and secondary focal segmental glomerulosclerosis (FSGS) from 1994 through 2013 in Olmsted County, Minnesota, and to identify the clinical and biopsy characteristics that distinguish primary from secondary FSGS.Patients and Methods
Olmsted County adult residents with native kidney biopsy from January 1, 1994, through December 31, 2013, and FSGS as the only glomerulopathy were identified. The clinical and pathologic characterstics of primary and secondary FSGS were described and compared, and incidence rates were calculated.Results
Of 370 adults biopsied, 281 had glomerular diseases, of which 46 (16%) had FSGS. From 1994-2003 to 2004-2013, there were significant increases in kidney biopsy rates (14.7 [95% CI, 12.1-17.3] vs 22.9 [95% CI, 20.0-25.7] per 100,000 person-years, 17% increase per 5 years; P<.001) and total FSGS rates (1.4 [95% CI, 0.6-2.2] vs 3.2 [95% CI, 2.1-4.3] per 100,000 person-years, 41% increase per 5 years; P=.02). Compared with patients with limited foot process effacement (<80%), patients with diffuse effacement (≥80%) without an identifiable cause had lower serum albumin levels (P<.001), had higher proteinuria (P<.001), and were more likely to have nephrotic syndrome (100% vs 4%; P<.001). Patients with diffuse effacement without an identifiable cause were classified as primary FSGS, which accounted for 3 of 12 patients (25%) during 1994-2003 and 9 of 34 (26%) during 2004-2013.Conclusion
Although the incidence of FSGS has increased, the proportions of primary and secondary FSGS have remained stable. 相似文献16.
Michele Carraro Cristina Zennaro Giovanni Candiano Luca Musante Maurizio Bruschi Gian Marco Ghiggeri Mary Artero Luigi Faccini 《Nephrology, dialysis, transplantation》2003,18(4):689-693
BACKGROUND: The putative humoral mediator thought to be involved in the pathogenesis of idiopathic nephrotic syndrome has not yet been identified. However, components exist in normal serum that block the permeability activity of FSGS serum in vitro. The potential of FSGS serum to increase glomerular albumin permeability may result from an imbalance between permeability factors and naturally occurring inhibitors. We hypothesized that this imbalance may be favoured by loss of inhibitory factors in nephrotic urine. METHODS: The study population consisted of seven patients with biopsy-proven FSGS, one with IgM nephropathy, and three with idiopathic nephrotic syndrome without biopsy, from whom frozen serum and dialysed and lyophilized urine samples were available.Glomerular albumin permeability (P(alb)) was determined from the change in glomerular volume induced by applying oncotic gradients across the basement membrane of normal isolated rat glomeruli pre-incubated with patient serum, normal control serum, patient serum mixed with an equal volume of urine from the same patient, or patient serum mixed with normal urine. Serum and urine apolipoproteins J and E were measured by dot-blot, utilizing peroxidase-labelled antibodies. The urinary capacity to scavenge oxygen radicals was determined after exposure of isolated glomeruli to superoxide generated by xanthine and xanthine oxidase. RESULTS: The mean P(alb) of the patients was markedly elevated at 0.74+/-0.08. The addition of urine from the same patient significantly reduced P(alb) (mean 0.15+/-0.23) in all but one of the patients with FSGS. Normal urine had no inhibitory effect in the 10 patients in which it was tested (mean 0.71+/-0.09). Serum apo J was slightly decreased and serum apo E was slightly increased compared with controls. Urine levels of both lipoproteins were significantly decreased compared with controls. Urine from FSGS patients effectively neutralized superoxide, whereas normal urine did not. CONCLUSIONS: Nephrotic urine but not normal urine contains components that block increased albumin permeability in isolated rat glomeruli induced by serum from patients with the idiopathic nephrotic syndrome. The inhibitory function of these components, which appear not to include apolipoproteins J and E, may involve scavenging of superoxide as a final common pathway. Loss in the urine from the serum of naturally occurring inhibitors in the initial stages of the disease may propagate proteinuria and glomerular injury. 相似文献
17.
M. Hattori Y. Akioka H. Chikamoto N. Kobayashi K. Tsuchiya M. Shimizu S. Kagami H. Tsukaguchi 《American journal of transplantation》2008,8(7):1550-1556
Recurrent focal segmental glomerulosclerosis (FSGS) is a major challenge in the field of transplantation. Integrin-linked kinase (ILK) has emerged as a key mediator of podocyte–glomerular basement membrane (GBM) interactions. To clarify the involvement of plasma factors in FSGS recurrence, we examined the effects of plasma from FSGS patients with or without posttransplant recurrence on cultured podocytes, focusing particularly on ILK activity. Podocytes from a conditionally immortalized mouse podocyte cell line were treated with plasma from 11 FSGS patients, and ILK activity was determined using an immune complex kinase assay. Treatment with plasma from three patients with recurrence induced an increase in ILK activity. In contrast, no increase in ILK activity was observed in cultured podocytes treated with plasma from the remaining three patients with recurrence and five patients without recurrence. Cultured podocytes treated with plasma that induced ILK activity showed alterations of focal contact and detachment from the laminin matrix. In conclusion, this preliminary study provides experimental evidence suggesting the possible presence of circulating toxic factors in the plasma of some patients with recurrent FSGS, which induce an increase in podocyte ILK activity that may lead to the detachment of podocytes from the GBM. 相似文献
18.
Abbas Madani Darioush Fahimi Rambod Taghaodi Fatemeh Mahjoob Niloofar Hajizadeh Behdad Navabi 《Iranian journal of pediatrics.》2010,20(2):199-205
Objective
Idiopathic Nephrotic syndrome (INS) is the most common form of nephrotic syndrome (NS) in children with the potential of progression to end stage renal disease (ESRD). INS is steroid-responsive in most children, but not all patients respond to it. The aim of this study was to determine the rate of steroid responsiveness in children with INS that referred to Children''s Medical Center since 1995 to 2007.Methods
In as a cross sectional study, the medical records of all children with INS aged 1 to 15 years who were referred to our referral hospital was reviewed. All patients with onset of disease less than 1 year of age, spontaneous remission, secondary forms of NS associated with systemic diseases, and follow up duration of less than 12 months were excluded from the study. Patients were categorized into 6 groups: Group 1 needed biopsy prior to any treatment, group 2 non-relapsing NS, group 3 infrequently relapsing NS, Group 4 frequently relapsing NS, group 5 steroid dependent NS and group 6 steroid resistant NS.Findings
A total of 238 patients were enrolled in the study. Kidney biopsy was performed in 79 cases. Minimal change lesion (MCL) was the most common (36.7%) pathological diagnosis. Steroid responsiveness was found in 81.5% of all cases including: 96% of MCL (consisting of biopsy proven cases and presumed ones), 32% of focal and segmental glomerulosclerosis, 73% of diffuse mesangial proliferation and 58% of membranoproliferative glomerulonephritis patients. During minimal follow up period of 12 months, there were 194 patients in remission, 32 patients with active NS, and 12 patients in ESRD.Conclusion
Our study results showed that 81.5% of all patients, 96.2% of MCL and 32% of FSGS patients initially responded to steroid therapy. 相似文献19.
20.
Berdeli A Mir S Yavascan O Serdaroglu E Bak M Aksu N Oner A Anarat A Donmez O Yildiz N Sever L Tabel Y Dusunsel R Sonmez F Cakar N 《Pediatric nephrology (Berlin, Germany)》2007,22(12):2031-2040
The podocin (NPHS2) gene encodes podocin protein, which has an important role in glomerular ultrafiltration and controlling slit membrane permeability.
The detection of an NPHS2 mutation affects the treatment plan for children with nephritic syndrome (NS). The frequency and spectrum of podocin mutations
in the Turkish population have remained largely unknown. The aim of this study was to screen for podocin mutations in Turkish
patients with steroid-resistant NS (SRNS) and to compare it with other published series. There were 295 children with SRNS,
originating from Turkey, included in this study. Forty-one patients (13.8%) had familial NS and 254 patients (86.2%) had sporadic
NS. Mutation analysis was performed in all eight exons of the NPHS2 gene with the direct DNA sequencing method. There were 53 different pathogenetic NPHS2 mutations detected, including 37 novel mutations. The mutation detection rate was 24.7% for all patients, 29.2% for familial,
and 24% for sporadic SRNS. The most common mutated exon was exon 5 (52 allele). The presence of mutations in exon 4 was found
to increase the risk of end-stage renal disease (ESRD). Among patients with mutations, the rates of renal failure and/or ESRD
(26%) were significantly higher than in those without mutations (12.6%). The mean time of progression to renal failure and
ESRD in patients with mutations (1.8 ± 2.5 years) was significantly shorter than in patients without mutations (3.7 ± 4.0 years).
Additionally, in patients with heterozygote mutations, fewer cases (13.6%) progressed to renal failure and/or ESRD than in
with patients who had homozygote/compound heterozygote mutations (31.3%). In conclusion, podocin mutations are responsible
for some of both familial and sporadic SRNS cases in Turkey. The mutations in this gene should be searched for in every child
after presentation with the first episode of NS. 相似文献